Pediatric Cardiology

, Volume 39, Issue 6, pp 1172–1180 | Cite as

Abnormalities in Left Ventricular Rotation Are Inherent in Young Children with Repaired Tetralogy of Fallot and Are Independent of Right Ventricular Dilation

  • Ruchika Karnik
  • Santosh C. Uppu
  • Meghan Tozzi
  • John Doucette
  • Irene D. Lytrivi
  • Miwa Geiger
  • Berthold Klas
  • Ira A. Parness
  • Rajesh Shenoy
  • Hari Rajagopal
  • Shubhika Srivastava
Original Article


Left ventricular (LV) dysfunction is a risk factor for adverse outcomes in older children and adults with repaired Tetralogy of Fallot (rToF). Pulmonary regurgitation (PR), right ventricular (RV) dilation, and dysfunction have been shown to result in abnormal LV myocardial mechanics and dysfunction. The aim of our study was to evaluate LV rotational mechanics, especially apical rotation in young children with rToF with and without RV dilation. This is a retrospective, single center study in 28 asymptomatic young children with rToF (16 with RV dilation; 12 without RV dilation); 29 age-matched normal controls. RV and LV systolic and diastolic function was studied using conventional two-dimensional echocardiography (2DE) and speckle tracking echocardiography (STE). Rotational mechanics studied included basal and apical rotation (BR, AR), peak twist (calculated by difference between the apical and basal rotation), twist rate (TR), and untwist rate (UnTR). The mean age of the cohort was 4.7 years (± 2.3). Abnormal AR, BR, TR, and UnTR were noted in patients with rToF. The abnormalities were significant in magnitude as well as the direction of rotation; more pronounced in the absence of RV dilation. LV systolic and diastolic dysfunction as evidenced by abnormal AR and degree of untwist is inherent in rToF and not associated with RV dilation in rToF children. Abnormal BR may reflect a lack of maturation to adult type of rotational mechanics. Further longitudinal studies are required to study the progression of these abnormalities and their correlation with clinical outcomes.


Repaired tetralogy of fallot Apical rotation Basal rotation Twist Untwist rate Biventricular function 



The authors would like to acknowledge Zhanna Roytman for data collection and Matthew’s Hearts of Hope Research Grant for financial support that was used towards statistical and Tomtec Software support.

Compliance with Ethical Standards

Conflict of interest

Mr. Klas is a full time employee of TomTec Imaging Systems. No disclosures for other authors.


  1. 1.
    Valente AM, Gauvreau K, Assenza GE, Babu-Narayan SV, Schreier J, Gatzoulis MA, Groenink M, Inuzuka R, Kilner PJ, Koyak Z, Landzberg MJ, Mulder B, Powell AJ, Wald R, Geva T (2014) Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of fallot enrolled in the INDICATOR cohort. Heart 100:247–253CrossRefPubMedGoogle Scholar
  2. 2.
    Geva T, Sandweiss BM, Gauvreau K, Lock JE, Powell AJ (2004) Factors associated with impaired clinical status in long-term survivors of tetralogy of fallot repair evaluated by magnetic resonance imaging. J Am Coll Cardiol 43:1068–1074CrossRefPubMedGoogle Scholar
  3. 3.
    Ghai A, Silversides C, Harris L, Webb GD, Siu SC, Therrien J (2002) Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of fallot. J Am Coll Cardiol 40:1675–1680CrossRefPubMedGoogle Scholar
  4. 4.
    Babu-Narayan SV, Diller GP, Gheta RR, Bastin AJ, Karonis T, Li W, Pennell DJ, Uemura H, Sethia B, Gatzoulis MA, Shore DF (2014) Clinical outcomes of surgical pulmonary valve replacement after repair of tetralogy of fallot and potential prognostic value of preoperative cardiopulmonary exercise testing. Circulation 129:18–27CrossRefPubMedGoogle Scholar
  5. 5.
    Knauth AL, Gauvreau K, Powell AJ, Landzberg MJ, Walsh EP, Lock JE, del Nido PJ, Geva T (2008) Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of fallot repair. Heart 94:211–216CrossRefPubMedGoogle Scholar
  6. 6.
    Friedberg MK, Fernandes FP, Roche SL, Grosse-Wortmann L, Manlhiot C, Fackoury C, Slorach C, McCrindle BW, Mertens L, Kantor PF (2012) Impaired right and left ventricular diastolic myocardial mechanics and filling in asymptomatic children and adolescents after repair of tetralogy of fallot. Eur Heart J Cardiovasc Imaging 13:905–913CrossRefPubMedGoogle Scholar
  7. 7.
    Dragulescu A, Friedberg MK, Grosse-Wortmann L, Redington A, Mertens L (2014) Effect of chronic right ventricular volume overload on ventricular interaction in patients after tetralogy of fallot repair. J Am Soc Echocardiogr 27:896–902CrossRefPubMedGoogle Scholar
  8. 8.
    Cheung EWY, Liang XC, Lam WWM, Cheung YF (2009) Impact of right ventricular dilation on left ventricular myocardial deformation in patients after surgical repair of tetralogy of fallot. Am J Cardiol 104:1264–1270CrossRefPubMedGoogle Scholar
  9. 9.
    Takayasu H, Takahashi K, Takigiku K, Yasukochi S, Furukawa T, Akimoto K, Kishiro M, Shimizu T (2011) Left ventricular torsion and strain in patients with repaired tetralogy of fallot assessed by speckle tracking imaging. Echocardiography 28:720–729CrossRefPubMedGoogle Scholar
  10. 10.
    Kim WJ, Lee BH, Kim YJ, Kang JH, Jung YJ, Song JM, Kang DH, Song JK (2009) Apical rotation assessed by speckle-tracking echocardiography as an index of global left ventricular contractility. Circ Cardiovasc Imaging 2:123–131CrossRefPubMedGoogle Scholar
  11. 11.
    Toumanidis ST, Kaladaridou A, Bramos D, Skaltsiotes E, Agrios JN, Vasiladiotis N, Pamboucas C, Kottis G, Moulopoulos SD (2013) Apical rotation as an early indicator of left ventricular systolic dysfunction in acute anterior myocardial infarction: experimental study. Hell J Cardiol 54:264–272Google Scholar
  12. 12.
    Park SJ, Miyazaki C, Bruce CJ, Ommen S, Miller FA, Oh JK (2008) Left ventricular torsion by two-dimensional speckle tracking echocardiography in patients with diastolic dysfunction and normal ejection fraction. J Am Soc Echocardiogr 21:1129–1137CrossRefPubMedGoogle Scholar
  13. 13.
    Burns AT, La Gerche A, Prior DL, MacIsaac AI (2009) Left ventricular untwisting is an important determinant of early diastolic function. JACC Cardiovasc Imaging 2:709–716CrossRefPubMedGoogle Scholar
  14. 14.
    Sengupta PP, Khandheria BK, Narula J (2008) Twist and untwist mechanics of the left ventricle. Heart Fail Clin 4:315–324CrossRefPubMedGoogle Scholar
  15. 15.
    Takahashi K, Al Naami G, Thompson R, Inage A, Mackie AS, Smallhorn JF (2010) Normal rotational, torsion and untwisting data in children, adolescents and young adults. J Am Soc Echocardiogr 23:286–293CrossRefPubMedGoogle Scholar
  16. 16.
    Notomi Y, Srinath G, Shiota T, Martin-Miklovic MG, Beachler L, Howell K, Oryszak SJ, Deserranno DG, Freed AD, Greenberg NL, Younoszai A, Thomas JD (2006) Maturational and adaptive modulation of left ventricular torsional biomechanics: Doppler tissue imaging observation from infancy to adulthood. Circulation 113:2534–2541CrossRefPubMedGoogle Scholar
  17. 17.
    Lopez L, Colan SD, Frommelt PC, Ensing GJ, Kendall K, Younoszai AK, Lai WW, Geva T (2010) Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr 23:465–495 (quiz 576–7)CrossRefPubMedGoogle Scholar
  18. 18.
    Valente AM, Cook S, Festa P, Ko HH, Krishnamurthy R, Taylor AM, Warnes CA, Kreutzer J, Geva T (2014) Multimodality imaging guidelines for patients with repaired tetralogy of fallot: a report from the AmericanSsociety of Echocardiography: developed in collaboration with the Society for Cardiovascular Magnetic Resonance and the Society for Pediatric Radiology. J Am Soc Echocardiogr 27:111–141CrossRefPubMedGoogle Scholar
  19. 19.
    Rudski LG, Lai WW, Afilalo J, Hua LQ, Handschumacher MD, Chandrasekaran K, Solomon SD, Louie EK, Schiller NB (2010) Guidelines for the echocardiographic assessment of the right heart in adults: a report from the american society of echocardiography endorsed by the european association of echocardiography, a registered branch of the european society of cardiology, and the canadian society of echocardiography. J Am Soc Echocardiogr 23:685–713CrossRefPubMedGoogle Scholar
  20. 20.
    Punn R, Behzadian F, Tacy TA (2010) Annular tilt as a screening test for right ventricular enlargement in patients with tetralogy of fallot. J Am Soc Echocardiogr 23:1297–1302CrossRefPubMedGoogle Scholar
  21. 21.
    Nagata Y, Takeuchi M, Mizukoshi K, Wu VCC, Lin FC, Negishi K, Nakatani S, Otsuji Y (2015) Intervendor variability of two-dimensional strain using vendor-specific and vendor-independent software. J Am Soc Echocardiogr 28:630–641CrossRefPubMedGoogle Scholar
  22. 22.
    Menting ME, Eindhoven JA, van den Bosch AE, Cuypers JAAE., Ruys TPE, van Dalen BM, McGhie JS, Witsenburg M, Helbing WA, Geleijnse ML, Roos-Hesselink JW (2014) Abnormal left ventricular rotation and twist in adult patients with corrected tetralogy of fallot. Eur Heart J Cardiovasc Imaging 15:566–574CrossRefPubMedGoogle Scholar
  23. 23.
    Sengupta PP, Tajik AJ, Chandrasekaran K, Khandheria BK (2008) Twist mechanics of the left ventricle principles and application. JACC Cardiovasc Imaging 1:366–376CrossRefPubMedGoogle Scholar
  24. 24.
    Ahmad N, Kantor PF, Grosse-Wortmann L, Seller N, Jaeggi ET, Friedberg MK, Mertens L (2012) Influence of RV restrictive physiology on LV diastolic function in children after tetralogy of fallot repair. J Am Soc Echocardiogr 25:866–873CrossRefPubMedGoogle Scholar
  25. 25.
    Fernandes FP, Manlhiot C, Roche SL, Grosse-Wortmann L, Slorach C, McCrindle BW, Mertens L, Kantor PF, Friedberg MK (2012) Impaired left ventricular myocardial mechanics and their relation to pulmonary regurgitation, right ventricular enlargement and exercise capacity in asymptomatic children after repair of tetralogy of fallot. J Am Soc Echocardiogr 25:494–503CrossRefPubMedGoogle Scholar
  26. 26.
    Weidemann F, Eyskens B, Mertens L, Dommke C, Kowalski M, Simmons L, Claus P, Bijnens B, Gewillig M, Hatle L, Sutherland GR (2002) Quantification of regional right and left ventricular function by ultrasonic strain rate and strain indexes after surgical repair of tetralogy of fallot. Am J Cardiol 90:133–138CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Ruchika Karnik
    • 1
  • Santosh C. Uppu
    • 1
  • Meghan Tozzi
    • 1
  • John Doucette
    • 2
  • Irene D. Lytrivi
    • 1
  • Miwa Geiger
    • 1
  • Berthold Klas
    • 3
  • Ira A. Parness
    • 1
  • Rajesh Shenoy
    • 1
  • Hari Rajagopal
    • 1
  • Shubhika Srivastava
    • 1
  1. 1.Division of Pediatric Cardiology, Department of PediatricsIcahn School of Medicine at Mount SinaiNew YorkUSA
  2. 2.Department of Environmental Medicine and Public HealthIcahn School of Medicine at Mount SinaiNew YorkUSA
  3. 3.TomTec Imaging Systems, GmbHMunichGermany

Personalised recommendations